Acquired factor V inhibitor with erythema and eosinophilia in a patient with end-stage renal disease

Abstract

Autoimmune factor V deficiency (AiFVD) is a rare bleeding disorder caused by factor V inhibitors. In this report, we present the case of an 89-year-old man who developed bleeding tendency during surgery to create arteriovenous fistula for hemodialysis. The bleeding tendency developed with prolongation of activated partial thromboplastin and prothrombin time, following drug-induced eruption and eosinophilia. Significant reduction in coagulation factor activity and inhibitory pattern in cross-mixing tests suggested the presence of inhibitors to coagulation factors. Subsequently, we detected a factor V inhibitor and anti-factor V autoantibodies was confirmed using enzyme-linked immunosorbent assay with purified human plasma factor V. Thus, the patient was ‘definitely diagnosed’ with AiFVD in accordance with the diagnostic criteria enacted by the Japanese Ministry of Health, Labor, and Welfare. The bleeding tendency improved after initiating oral prednisolone 50 mg (1 mg/kg) followed by normalization of activated partial thromboplastin time and prothrombin time at the 34th day. After improving the coagulation system prolongation, the inhibitor and autoantibodies has been eradicated. Since it is suggested that drug-induced immune response can cause AiFVD, AiFVD should be considered in patients who undergo hemodialysis and develop failure of hemostasis and drug-induced eruption.

A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on Japan

“…Among them, 24 patients were diagnosed with AiFVD due to the presence of anti-FV autoantibodies detected using immunological assays (e.g., immunoblot, immunochromatography, and enzyme-linked immunosorbent assay) and FV inhibitors detected using functional coagulation tests (e.g., Bethesda assay and FV mixing test). 11 12 13 14 15 16 17 Our research results do not include cases in which the bovine thrombin preparation produced an FV inhibitor due to an anti-bovine FV antibody, since the bovine thrombin preparation was no longer commercially available in Japan at that period. Based on a population size of 125 million individuals as of August 2021 ( ), the estimated incidence of AiFVD in Japan is at least approximately 0.04 per million persons per year.…”